Chelation Therapy

Diagnosis

Tests to Monitor Iron Overload

There are various tests used to monitor iron levels, including:

Ferritin Level: The amount of iron in the body can be checked with a blood test called a serum ferritin. This test will give a general idea of how much iron is in the body. Acceptable ferritin levels are generally less than 1000 – 1500 ng/mL .

SQUID Test: SQUID is a special type of x-ray that uses magnets to show much iron is in the body. This test is very accurate and non-invasive. The SQUID test requires the patient to lie still for a ten to fifteen minute procedure. The SQUID test is currently only available in two locations in the United States: Columbia Presbyterian Medical Center in New York City and Children’s Hospital Oakland in Oakland, California.For more information on the SQUID test, see:http://www.childrenshospitaloakland.org/healthcare/depts/Ferritometer.asp

T2* MRI and FerriScan MRI: These tests are special types of MRIs. The T2* MRI measures the amount of iron in the heart, while the FerriScan MRI measures the amount of iron in the liver. These tests are considered to be very accurate and are non-invasive. These tests are relatively new and not available at every medical center. To find locations which offer the FerriScan MRI, see:http://www.resonancehealth.com/ and www.ferriscan.com.

Liver Biopsy: A liver biopsy is an accurate, though invasive test to measure the amount of iron in the liver. Patients are typically sedated during the biopsy. The biopsy consists of inserting a needle into the liver and removing a small piece of liver tissue. This tissue is then tested to determine the amount of iron present. The liver tissue can also be analyzed to see if the iron has caused any damage to the liver, such as fibrosis or cirrhosis.

Chelation Therapy

Iron overload can be prevented and treated with chelation therapy. Chelation therapy refers to using medication to remove excess metals, such as iron, from the body. As the body has no natural way of removing excess iron from the body, chelation therapy is the only way to remove the iron. Therefore, chelation therapy is a critical component to the health and well being of a DBA patient receiving chronic blood transfusions.

There are currently two chelation drugs available in the United States: Exjade (Defarasirox) and Desferal (Deferoxamine).

Exjade: Exjade is a relatively new chelation drug here in the United States, as it has only been available since 2005. Exjade is an oral medication. Exjade tablets are dissolved in water or juice and taken once a day on an empty stomach, preferably at the same time every day. Exjade works by binding to the iron and removing the iron from the body through the stool. Exjade may be taken by patients age two years and older. For more information on Exjade, see: http://www.us.exjade.com/index.jsp

Desferal: Desferal is a chelation drug that has been used in the United States for many years. Desferal cannot be taken by mouth, but must be given as an infusion. This means that desferal enters the body through a needle that is placed under the skin (subcutaneously). Desferal is usually given over 8-12 hours, 5-7 nights per week using a battery operated pump. Many patients do their desferal treatments at night, while they are sleeping. Desferal works by removing the iron from the body through the urine. Patients using desferal will notice their urine is orange or red colored, which means the medication is working.

The vast majority of patients who are on chronic blood transfusion therapy and who chelate properly will have long, normal lives. Some patients may have unique issues that cause them to accumulate iron. Patients should consult with their individual doctors, have a program for monitoring iron levels, and carefully follow doctor’s orders regarding proper chelation.